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Paeds SAQscardiology

Paeds SAQs · cardiology

Cyanotic newborn and critical congenital heart disease screening — formative SAQs

Formative SAQs on the cyanotic newborn and critical congenital heart disease screening: applying the pulse-oximetry thresholds, starting prostaglandin E1 before the echocardiogram, separating cyanotic congenital heart disease from pulmonary and sepsis mimics, and escalating to a cardiac centre.

20 marks30 min
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Target exams

RACP General PaediatricsMRCPCH ClinicalRACP DWE

Target exams

RACP General PaediatricsMRCPCH ClinicalRACP DWE
Prompt
Critical congenital heart disease screening and the cyanotic newborn

SAQ 1 (10)

A term infant born after an uncomplicated pregnancy is noted on the postnatal ward at thirty hours of age to be dusky during a feed but otherwise comfortable, with no respiratory distress. The midwife records a pulse oximetry of 86 per cent in the right hand and 84 per cent in the foot in air. There is no murmur. [2] [3]

a) Interpret these pulse-oximetry findings against the screening thresholds, and state the immediate next step. (2 marks) [2]

b) The baby is alert and looks well. Justify why this appearance does not lower your concern, and name the bedside examination findings that would localise a cardiac lesion. (3 marks) [2]

c) Outline your immediate management, naming the drug, the dose and the airway consideration, and explain why it precedes the echocardiogram. (3 marks) [8] [9]

d) The team asks whether to give 100 per cent oxygen while awaiting the team. Explain the principle that governs oxygen use in a suspected duct-dependent mixing lesion. (2 marks) [2]

SAQ 2 (10)

A two-day-old infant is brought to the emergency department grey, mottled and tachypnoeic, with weak femoral pulses and a capillary refill of five seconds. The blood gas shows a pH of 7.10 with a lactate of 8 mmol per litre. The baby was discharged home well at twenty-four hours and had no antenatally detected anomaly. [1] [8]

a) Give a problem representation and state the most likely physiological category of lesion, with your reasoning. (2 marks) [2]

b) Outline the resuscitation in order, naming the specific infusion that reopens the duct, its starting dose range, and the monitoring required. (3 marks) [9] [8]

c) Discuss the differential diagnosis of neonatal cyanosis and the role of the hyperoxia test and the echocardiogram in resolving it, including the limits of the hyperoxia test. (3 marks) [2]

d) Explain why this presentation represents a failure of the screening programme, and state the layered screening that should have detected the lesion earlier. (2 marks) [1]

References

  1. [1]de-Wahl Granelli A, Wennergren M, Sandberg K, et al. Impact of pulse oximetry screening on the detection of duct dependent congenital heart disease: a Swedish prospective screening study in 39,821 newborns. BMJ, 2009.PMID 19131383
  2. [2]Mahle WT, Newburger JW, Matherne GP, et al. Role of pulse oximetry in examining newborns for congenital heart disease: a scientific statement from the American Heart Association and American Academy of Pediatrics. Circulation, 2009.PMID 19581492
  3. [3]Ewer AK, Middleton LJ, Furmston AT, et al. Pulse oximetry screening for congenital heart defects in newborn infants (PulseOx): a test accuracy study. Lancet, 2011.PMID 21820732
  4. [8]Aykanat A, Yavuz T, Özalkaya E, et al. Long-Term Prostaglandin E1 Infusion for Newborns with Critical Congenital Heart Disease. Pediatr Cardiol, 2016.PMID 26260095
  5. [9]Vari D, Xiao W, Behere S, et al. Low-dose prostaglandin E1 is safe and effective for critical congenital heart disease: is it time to revisit the dosing guidelines? Cardiol Young, 2021.PMID 33140712